Forny

Choledochal
DOI:
10.1590/0100-69912014005006
cyst in childhood: review of 30 cases

331
Original Article

Choledochal cyst in childhood: review of 30 cases

Cisto de coldoco na infncia: reviso de 30 casos
DANIELLE NUNES FORNY1; SAULO MARCOS REBELLO FERRANTE2; VINCIUS GOMES DA SILVEIRA,TCBC-RJ2; IVONETE SIVIERO2; VERA LUCIA ANTUNES
CHAGAS3; IVENS BAKER MIO2

A B S T R A C T
Objective
Objective: To analyze and discuss the clinical data, diagnosis and treatment of a number of patients with cystic dilatation of the common
bile duct of a Brazilian pediatric hospital. Methods
Methods: We analyzed 30 patients treated at the Martago Gesteira Institute of Pediatrics and
Child Care of the Federal University of Rio de Janeiro for 23 years ,with statistical analysis of epidemiological data, clinical manifestations,
diagnosis, treatment and postoperative outcome. Results
Results: We observed a marked female predominance (73.4% of cases), the diagnosis
being made in the first decade of life in 90% of patients. The most prevalent clinical manifestation was jaundice (70% of cases) and the
classic triad of choledochal cyst was not observed. Abdominal ultrasound was the first imaging examination performed, with a sensitivity of
56.6%, with diagnostic definition in 17 children. Two patients (6.6%) had prenatal diagnosis. All patients underwent surgical treatment,
cyst resection with Roux-en-Y hepaticojejunostomy being performed in 80% of cases. The incidence of postoperative complications was
13.3% and the mortality rate was 6.6%, ie two patients were diagnosed with Carolis disease. Conclusion
Conclusion: The non-observance of the
classic triad of choledochal cyst suggests that its incidence is lower than that reported in the medical literature. The surgical treatment of
choledochal cysts, with resection and bilioenteric anastomosis, is safe even for small children.
Key words: Choledochal cyst. Jaundice. Bile Ducts. Anastomosis, Roux-en-Y. Cholangiocarcinoma. Child.

INTRODUCTION

he choledochal cysts consist of congenital abnormalities,

with dilations of intra and / or extrahepatic bile ducts.
Its occurrence is 1: 100,000 to 1:150,000 live births in the
West and 1:1,000 in Asia1. They are more common in
females in the ratio 3:1 to 4:1 1,2. While they may manifest
in adulthood, about 60% of cases are diagnosed in the first
decade of life3. The most common diagnostic method is
ultrasound. Currently, the recommended treatment is
resection of the cyst or, when technically difficult, of the
mucosa due to the high risk of late malignancy
transformation1,4. Despite its low incidence, the choledochal
cyst should be researched in children with suggestive
symptoms, preventing the development of biliary cirrhosis
or cancer of the biliary tract.
We present a series of 30 patients, discussing
the epidemiological, anatomical classification, diagnostic
and therapeutic methods, and correlating these data with
the evolution and literature.

METHODS

Care IPPMG of the Federal University of Rio de Janeiro

UFRJ for a period of 23 years. From an initial group of
35 patients, five were excluded. Exclusion criteria were:
divergence of data (one patient), transfer to another hospital for surgical treatment (one patient) and insufficient
postoperative follow-up (less than three outpatient visits
three patients). For inclusion in the study we considered
patients of both genders, aged between one month and
13 incomplete years, surgery performed at the Department
of Pediatric Surgery of IPPMG and attending at least three
visits postoperatively. We analyzed the main clinical
manifestations, age at which the diagnosis was made,
exams, applied surgical procedures, complications,
anatomical classification of cysts, histopathology of the liver
and resected cysts, and postoperative outcome, with
descriptive statistical analysis by calculating mean, median,
standard deviation and percentage.
The project was approved by the Ethics in Research
Committee of the Martago Gesteira Institute of Pediatrics
and Child Care, Federal University of Rio de Janeiro, under
the number 291,124, CAAE 16047813.3.0000.5264.

RESULTS

We conducted a retrospective analysis of 30

patients with choledochal cyst attended at the Pediatric
Surgery, Martago Gesteira Institute of Pediatrics and Child

Eight patients (26.6%) were male and 22

(73.4%) female. The average age of onset of symptoms

1. Martago Gesteira Institute of Pediatrics and Child Care (IPPMG), Federal University of Rio de Janeiro UFRJ; 2. Department of Surgery,
Faculty of Medicine, UFRJ; 3. Department of Pathology, UFRJ.
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was 37.5 months ( 28.2), with a median of 31.5 months,

ranging from 4 to 100.
The most common clinical manifestation was
jaundice in 70% of cases (21 patients), followed by abdominal pain, present in 46.6% of patients (14 cases). Acute
cholangitis was present in one child (3.3%). Four children
(13.3%) were asymptomatic at the first surgical evaluation:
two had prenatal diagnosis by obstetric ultrasonography,
and two were evaluated due to hepatomegaly. One patient
had a palpable abdominal mass (3.3%) (Figure 1). The
classic triad of choledochal cyst (abdominal pain, jaundice,
and a palpable mass) was not observed.
Ultrasonography (USG) of the abdomen was done
in 30 patients. Of these, only 17 were confirmed in this first
evaluation, with a sensitivity of 56.6%. In other tests,
diagnoses were varied: choledocholithiasis (2), cyst of head
of pancreas (1), pancreatic pseudocyst (1), hepatomegaly
(1) and ascariasis of the biliary tract (1). In seven patients,
we observed dilation or ectasia of the main bile duct, without
being characterized as choledochal cyst. Two patients were
diagnosed by prenatal ultrasonography in the third trimester
of pregnancy. The ultrasound confirmation was made in
the first and 23 months. In five patients computed
tomography (CT) of the abdomen was indicated,
demonstrating choledochal dilatation in three, with diagnosis
in two, with a sensitivity of 40%. Magnetic resonance
imaging (MRI) was used in nine patients, being diagnostic
in all cases, with a 100% sensitivity. It was indicated after
abdominal ultrasonography in seven cases and after abdominal CT in two. In one patient the radiological diagnosis
was made by percutaneous cholangiography and in another,
by intravenous cholangiography.
The average age of diagnosis was 47.4 months
( 36.7), ranging from one to 129, with a median of 36.5.
Ninety percent of patients were less than ten years old.

Figure 1

The 30 patients underwent surgical treatment, at mean

age of 50.2 months, and median 40.5 ( 38), ranging from
three to 130 months. The mean interval between diagnosis
and surgery was 2.9 months.
The common bile duct cyst type I, according to
the Alonso-Lej classification5 modified by Todani6, was
observed in 93.4% of patients (28 patients), followed by
cyst type V ( diffuse Carolis disease) in 6.6% (two patients).
In 24 patients (80%) we performed resection of
the choledochal cyst and Roux-en-Y hepaticojejunostomy.
In five patients (16.6%) we applied the Lilly technique,
with resection of the choledochal mucosa and Roux-en-Y
hepaticojejunostomy. In one patient with Carolis disease
we performed only a liver biopsy (Table 1). The removal of
liver tissue for histological analysis was performed in 23
patients of the series (76.6%).
Four patients (13.3%) had immediate
postoperative complications: one had choleperitoneum due
to partial dehiscence of the biliodigestive anastomosis, which
required reoperation, but progressed satisfactorily; three
patients had acute cholangitis, two of them had Carolis
disease.
In eight patients (26.6%) the liver had a cirrhotic
aspect at laparotomy. None of the excised cysts had
malignant degeneration or metaplasia at histopathology.
Ninety percent of liver biopsies demonstrated microscopic
structural abnormalities, hepatic fibrosis being the most
common, present in 45.4% of samples, including the two
patients who had had prenatal diagnosis. Cirrhosis was
observed in 13.6%. Two pathological examinations were
normal (Table 2).
The mean duration of postoperative follow-up
was 35.1 months ( 30.4), ranging from three to 104. In
this period, two patients with Carolis disease died, showing
a mortality rate of 6.6%. Twenty-eight patients showed

Clinical manifestations.
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clinical improvement, without jaundice nor signs of liver

dysfunction, and appropriate development for the age.

DISCUSSION
Choledochal cysts are congenital malformations
of bile ducts that represent a major diagnostic and
therapeutic challenge for the pediatric surgeon, demanding
a high degree of suspicion and efficient etiological
investigation for the correct diagnosis and institution of
appropriate treatment. Treatment should consist of
resection of the dilated portion of the extrahepatic biliary
reconstruction with a Roux-en-Y hepaticojejunostomy. When
intrahepatic dilatation is very extensive, liver transplantation
is an alternative.
The observation of the predominance of female
patients in this sample, consisting of 73.4%, is consistent
with other studies on choledochal cysts1,5,6. The age at which
the diagnosis was made, with the majority of patients in
the first decade of life, agrees with other reports3,7, where
80% of cases are diagnosed before ten years of life, whereas
only 25% of common bile duct cysts are seen in the first
year2.
There is no consensus in the literature about the
most common clinical presentation. Some authors3,8 believe
jaundice to be the main signal, similar to the result obtained
in this sample. Other studies 2,9, however, report that abdominal pain is the most prevalent symptom. Lilly argued that
jaundice should be more commonly seen in infants and
abdominal pain in older patients, probably due to the better
capacity to verbalize10. Though abdominal pain, jaundice
and abdominal mass are described as the classic triad of
choledochal cyst5, their association was not observed in
this study, in disagreement with data reported by other
authors3,7,11. Although the concept that this presentation
would be more frequent in children than in adults 7, it is
believed that early diagnosis in the current era would prevent
the choledochal cyst from greatly increasing in volume,
explaining the absence of an abdominal mass.
Currently, the preoperative diagnosis of
choledochal cyst is mostly done by the USG, which has
high sensitivity for the diagnosis of biliary tract disease 12,13.
It was the first complementary method used in the 30
patients, however, there was a sensitivity of only 56.6% in
this sample, with a large number of tests being carried out
by radiologists not specialized in pediatric diseases. This
Table 1 -

reinforces the idea that both clinicians, radiologists and

surgeons require extensive knowledge of this malformation
for the diagnosis to be established. Prenatal diagnosis of
choledochal cyst is possible from the second trimester of
pregnancy 14, as noted in two children, and should always
be confirmed after birth.
Although abdominal CT was performed in five
patients, only in two the diagnosis was established. The
low sensitivity is probably due to the low resolution of the
images obtained in the first two patients, in the 80s, one
with suspected choledocholithiasis and another with
suspected cyst of head of pancreas. They were submitted
to percutaneous cholangiography and venous
cholangiography, respectively. These diagnostic modalities
are no longer used, since they could be replaced by less
invasive and more sensitive radiological methods 1. On the
other hand, in all nine patients who underwent MRI the
diagnosis was correctly achieved, demonstrating that this
method is a more sensitive diagnostic tool for the evaluation
of diseases of the biliary tract than CT 15. More recently,
the three-dimensional reconstruction of MRI
cholangiopancreatography allows better anatomical
evaluation of biliary tract and pancreas. Another innovation
is the virtual cholangioscopy, which allows preoperative
radiological exploration of the bile ducts 16.
Roux-en-Y hepaticojejunostomy was performed
in 24 patients, and biliodigestive anastomosis associated
with resection of the mucosal lining of the cyst, the Lilly
technique, was performed in five patients. In this series,
the low incidence of postoperative complications and good
clinical outcome suggests that it is technically possible to
safely perform biliodigestive anastomoses in children, as
previously described 17. One patient with Carolis disease
Table 2 -

Hepatic Histopathological changes.

Histopathological changes

Liver fibrosis
Ductular proliferation
Cholestasis
Perisinusoidal fibrosis
Fibrosis progressing to cirrhosis
Fibrosis progressing to cirrhosis
Hyperplasia of sinusoidal cells
Minimal changes
Normal

10
6
5
3
3
2
2
2
2

%
45.4
27.3
22.7
13.6
13.6
9
9
9
9

Surgical procedures.

Surgical procedure
Roux-en-Y hepaticojejunostomy
Lillys Surgery
Liver biopsy (isolated)
Total

Patients (N)
24
5
1
30
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%
80
16.7
3.3
100

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was treated in the 80s with palliative Roux-en-Y

hepaticojejunostomy, since it would not be possible to
perform liver transplantation in that period. In another
patient with the same diagnosis due to advanced liver
cirrhosis, it was decided only by liver biopsy, without
biliodigestive derivation.
According to medical literature1,3,5, choledochal
cysts type I correspond to up to 95% of all cases, confirming
the demonstrated in this series, where the majority of cysts
(93.3%) represented a fusiform dilatation of the common
bile duct.
The observed postoperative complications may
be considered uncommon. The dehiscence of the
biliodigestive anastomosis is infrequent1,18; we observed it
in only one case, which was reoperated, with good
evolution. The incidence of postoperative cholangitis was
10%, agreeing with 8%19 to 10%20 reported in the literature.
The highest incidence of cholangitis in this series can be
explained because two of the three affected patients had
Carolis disease, which leads to biliary stasis, increasing
the possibility of infection. The incidence of cholangitis in
patients with Carolis disease can reach 60%21.
Although there are few reports on the microscopic
abnormalities of the liver of patients with choledochal cyst,
some studies claim that most patients, even those with
prenatal diagnosis or operated in the first year of life, have
changes in their liver biopsies as a result of biliary
obstruction, with ductular proliferation, fibrosis and even
cirrhosis22-24, agreeing with our results, since only two liver
histopathology exams were normal. Not even the patients
treated before one year of age showed normal liver
histology. This reinforces the need for early diagnosis and

treatment to prevent further damage to the liver

parenchyma.
Postoperative monitoring demonstrated that the
mortality rate was 6.6%, two patients with type V cysts, a
higher incidence than that of Miyano et al.25, with zero
mortality in a analysis of 180 cases in 30 years, and of
Hung et al.17, also without mortality in a group of 25 patients
followed for 20 years. Those authors do not report on their
analyses the individuals affected by Carolis disease, which
usually evolve into irreversible liver failure, when resection
of the affected segment or liver transplantation are not
possible 26. Transplanted patients have good postoperative
outcome, preventing the development of
cholangiocarcinoma 27.
Given all these data, we conclude that jaundice
in childhood must remain a warning sign for congenital
malformations of the biliary tract. The lack of observation
of the classic triad of choledochal cyst in the studied patients
suggests that its incidence in childhood is lower than that
reported in the medical literature. The surgical treatment
of choledochal cysts, with resection and
hepaticojejunostomy, is the treatment of choice and is safe
even in young children. Diagnosis and treatment should be
early to avoid a greater involvement of the hepatic
parenchyma, whose severity depends on the degree of
obstruction and time course.
Acknowledgements
To Rosangela Martins, Biostatistician of the
Research Division of the Clementino Fraga Filho University
Hospital HUCFF, UFRJ, for the statistical analysis of this
work.

R E S U M O
Objetivo: analisar e discutir os dados clnicos, o diagnstico e tratamento de uma srie de pacientes portadores de dilataes csticas
Objetivo
Mtodos: foram analisados 30 pacientes tratados no Instituto de Pediatria e
do coldoco de um hospital peditrico brasileiro. Mtodos
Puericultura Martago Gesteira da Universidade Federal do Rio de Janeiro durante 23 anos, com anlise estatstica de dados
Resultados: foi observada marcada
epidemiolgicos, manifestaes clnicas, diagnstico, tratamento e evoluo ps-operatria. Resultados
predominncia do sexo feminino (73,4% dos casos), sendo o diagnstico feito na primeira dcada de vida em 90% dos pacientes. A
manifestao clnica mais prevalente foi a ictercia (70% dos casos) e a trade clssica do cisto de coldoco no foi observada. A
ultrassonografia abdominal foi o primeiro exame de imagem realizado, demonstrando sensibilidade de 56,6%, com definio
diagnstica em 17 crianas. Dois pacientes (6,6%) tiveram diagnstico pr-natal. Todos os pacientes foram submetidos a tratamento
cirrgico, sendo a resseco do cisto com hepaticojejunostomia com Y de Roux realizada em 80% dos casos. A incidncia de
complicaes ps-operatrias foi 13,3% e a taxa de mortalidade foi 6,6%, ou seja, dois pacientes com diagnstico de doena de
Concluso: a no observao da trade clssica do cisto de coldoco nos pacientes avaliados sugere que a sua incidncia seja
Caroli. Concluso
menor que a relatada na literatura mdica mundial. O tratamento cirrgico dos cistos de coldoco, com sua resseco e anastomose
bleodigestiva seguro, mesmo em crianas pequenas.
Descritores: Cisto do Coldoco. Ictercia. Ducto Biliar. Anastomose em Y de Roux. Colangiocarcinoma. Criana.
Descritores

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Received on 12/12/2013
Accepted for publication 05/02/2014
Conflict of interest: none.
Source of funding: none.
Mailing address:
Danielle Nunes Forny
E-mail: dforny@gmail.com

Rev. Col. Bras. Cir. 2014; 41(5): 331-335